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Nebulin Rabbit pAb (bs-2401R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-2401R
英文名称 Nebulin Rabbit pAb
中文名称 伴肌动蛋白抗体
别    名 DKFZp686C1456; FLJ11505; FLJ36536; FLJ39568; FLJ39584; NEB 177D; NEB; NEB177D; NEM 2; NEM2; NEBU_HUMAN; FLJ11505.  
研究领域 细胞生物  免疫学  结合蛋白  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 (predicted: Human,Mouse,Rat,Rabbit,Pig,Cow,Chicken,Dog)
产品应用 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 500-800 kDa
检测分子量
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Nebulin: 6401-6669/6669 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 Nebulin is a high molecular weight protein (500-800 kD), localized at the N2-line of skeletal muscle myofibrils. It exhibits an elastic stretch dependence, as it maintains the same proportional distance between the z-line and the m-line as the sarcomere is stretched.

Function:
This giant muscle protein may be involved in maintaining the structural integrity of sarcomeres and the membrane system associated with the myofibrils. Bind and stabilize F-actin.

Subcellular Location:
Cytoplasm > myofibril > sarcomere. Cytoplasm > cytoskeleton.

Tissue Specificity:
Muscle specific. Located in the thin filament of striated muscle.

DISEASE:
Defects in NEB are the cause of nemaline myopathy type 2 (NEM2) [MIM:256030]. A form of nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-or rod-like structures in muscle fibers on histologic examination.

Similarity:
Contains 178 nebulin repeats.
Contains 1 SH3 domain.

SWISS:
P20929

Gene ID:
4703

Database links:

Entrez Gene: 4703 Human

Entrez Gene: 17996 Mouse

Entrez Gene: 311029 Rat

Omim: 161650 Human

SwissProt: P20929 Human

Unigene: 588655 Human



具有稳定细肌丝的作用。
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